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Vol. 52. Núm. 6.
Páginas 309-320 (Julio 2005)
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Vol. 52. Núm. 6.
Páginas 309-320 (Julio 2005)
Notas clínicas
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Feocromocitoma
Pheochromocytoma
Visitas
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E. Delgadoa,??
Autor para correspondencia
eliasdelgado@telefonica.net

Correspondencia: Dr. E. Delgado Álvarez. Servicio de Endocrinología y Nutrición. Hospital Universitario Central de Asturias. Celestino Villamil, s/n. 33006 Oviedo. Asturias. España.
, P. Botasb
a. Servicio de Endocrinología y Nutrición. Hospital Universitario Central de Asturias. Oviedo. Asturias. España
b. Servicio de Endocrinología y Nutrición. Hospital San Agustín. Avilés. Asturias. España
Información del artículo
Resumen
Bibliografía
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Estadísticas

El feocromocitoma es un tumor productor de catecolaminas que se origina en las células cromafines de la médula suprarrenal. Supone menos del 0,2% de todos los pacientes hipertensos, pero la rareza del tumor no resta importancia a su gravedad, ya que el correcto diagnóstico y el adecuado tratamiento suelen conseguir su curación, mientras que errores diagnósticos o su inadecuado tratamiento puede tener consecuencias fatales. Es la urgencia vital de causa endocrinológica más grave. En la actualidad un porcentaje importante de feocromocitomas (el 10-49%, según las series) se diagnostica en el estudio de un incidentaloma suprarrenal.

La clínica que origina es de una gran variabilidad, desde personas asintomáticas hasta la muerte por crisis hipertensiva grave con fallo multiorgánico como primera manifestación, aunque lo más típico es la tríada compuesta por paroxismos de cefalea, sudación y taquicardia. El diagnóstico se debe sospechar ante clínica compatible y la confirmación se realiza al demostrar una hiperproducción de catecolaminas y sus metabolitos, fundamentalmente al objetivar una eliminación urinaria aumentada.

La tomografía axial computarizada y la resonancia magnética nuclear nos permitirán localizar el tumor y, en caso de duda, la gammagrafía con meta-yodo-bencilguanidina, que tiene una gran especificidad.

El tratamiento es la resección del tumor, siempre que sea posible, previa preparación farmacológica con bloqueo alfaadrenérgico y, si es preciso, betaadrenérgico, iniciado posteriormente durante 2-3 semanas. En los casos de feocromocitomas malignos en los que no se pueda realizar la resección se realizará tratamiento farmacológico con alfa-metil-tirosina o bien dosis terapéuticas de 131I-MIBG.

Palabra clave:
Feocromocitoma
Tumor
Catecolaminas

Pheochromocytoma is a catecholamine-producing neoplasm originating in chromaffin cells, mostly situated within the adrenal medulla.

This tumor occurs in less than 0.2% of patients with hypertension. Although rare, it must be considered in the differential diagnosis because accurate diagnosis and appropriate treatment usually achieve a cure whereas diagnostic errors or inappropriate treatment can have fatal consequences. It is the most serious endocrinological emergency.

Currently, a considerable percentage of pheochromocytomas (10-49% depending on the series) is diagnosed during investigation of adrenal incidentalomas. Clinical presentation is highly variable, ranging from asymptomatic forms to death secondary to severe hypertensive crisis with multiorgan failure as the first manifestation. However the typical presentation is the classic triad of episodic headache, sweating and tachycardia. The diagnosis should be suspected in patients with compatible clinical features and is confirmed by a finding of hyperproduction of catecholamines and their metabolites, demonstrated by increased urinary excretion.

Computed tomography and magnetic resonance imaging allow localization of the neoplasm and in difficult cases meta-iodobenzyl- guanidine (MIBG) scintigraphy has high specificity.

Treatment consists of surgical removal of the tumor with previous medical preparation using alpha-adrenergic blockade and, if necessary, subsequent beta-adrenergic blockade for 2-3 weeks. Treatment options in malignant pheochromocytomas that cannot be surgically resected are drug therapy with alpha-methyl-tyrosine or therapeutic doses of 131I-MIBG.

Key words:
Pheochromocytoma
Neoplasm
Catecholamines
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Bibliografía
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